# Pediatric Hepatobiliary Lithiasis in Homozygous Sickle Cell Disease: Laboratory Insights Into Cholestasis and Hemolysis

**Authors:** Faralahy H Rakotonjafiniarivo, Tahianasoa Randriamampianina, Stephania Niry Manantsoa, Miora Koloina Ranaivosoa, Aimée O Rakoto Alson

PMC · DOI: 10.7759/cureus.102913 · 2026-02-03

## TL;DR

A 13-year-old girl with sickle cell disease developed liver and gallbladder issues, showing how hemolysis and cholestasis markers can help diagnose these complications early.

## Contribution

Highlights the diagnostic utility of combining hemolysis and cholestasis markers in pediatric sickle cell disease patients.

## Key findings

- Severe anemia and reticulocytosis indicated active hemolysis.
- Mixed hyperbilirubinemia and elevated CRP suggested cholestasis and inflammation.
- Imaging confirmed microlithiasis without bile duct obstruction.

## Abstract

Chronic hemolysis in sickle cell disease (SCD) predisposes patients to hepatobiliary complications, including pigment gallstones. We report a 13-year-old girl with hemoglobin SS SCD who presented with right upper quadrant pain, progressive jaundice, and fever. Laboratory evaluation revealed severe anemia with reticulocytosis, mixed hyperbilirubinemia, elevated lactate dehydrogenase, normal cholestatic enzymes, and marked C-reactive protein elevation. Imaging confirmed microlithiasis without significant bile duct obstruction. Supportive care included transfusion, hydration, antibiotics, and pain control. Elective cholecystectomy was planned after stabilization. This case underscores the diagnostic value of combined hemolysis and cholestasis markers for early recognition of hepatobiliary complications in pediatric patients with SCD.

## Linked entities

- **Diseases:** sickle cell disease (MONDO:0011382), cholestasis (MONDO:0001751)

## Full-text entities

- **Genes:** GGTLC5P (gamma-glutamyltransferase light chain 5 pseudogene) [NCBI Gene 653590] {aka GGT}, ALPP (alkaline phosphatase, placental) [NCBI Gene 250] {aka ALP, PALP, PLAP, PLAP-1}, GPT (glutamic--pyruvic transaminase) [NCBI Gene 2875] {aka AAT1, ALT, ALT1, GPT1, SGPT}, SLC17A5 (solute carrier family 17 member 5) [NCBI Gene 26503] {aka AST, ISSD, NSD, SD, SIALIN, SIASD}, CRP (C-reactive protein) [NCBI Gene 1401] {aka PTX1}, GGT1 (gamma-glutamyltransferase 1) [NCBI Gene 2678] {aka CD224, D22S672, D22S732, GGT, GGT 1, GGTD}
- **Diseases:** hepatitis A, B, and C (MESH:D006509), abdominal pain (MESH:D015746), Gallstone (MESH:D042882), inherited hemoglobinopathy (MESH:D006453), pain (MESH:D010146), vaso-occlusive crises (MESH:D013224), primary liver disease (MESH:D008107), inflammatory (MESH:D007249), Accelerated red blood cell destruction (MESH:C562718), Chronic hemolysis (MESH:D006461), duct (MESH:D001649), acute cholangitis (MESH:D000208), fever (MESH:D005334), reticulocytosis (MESH:D045262), microlithiasis (MESH:C566478), jaundice (MESH:D007565), Hepatobiliary Lithiasis (MESH:D020347), hemorrhagic (MESH:D006470), splenomegaly (MESH:D013163), leukocytosis (MESH:D007964), Hepatobiliary complications (MESH:D004066), pigment stones (MESH:D007669), Cholestasis (MESH:D002779), thrombocytopenia (MESH:D013921), hepatomegaly (MESH:D006529), Viral hepatitis (MESH:D014777), vaso-occlusive crisis (MESH:D001157), hemoglobin SS (MESH:D006445), anemia (MESH:D000740), rigidity (MESH:D009127), hyperbilirubinemia (MESH:D006932), SCD (MESH:D000755), biliary obstruction (MESH:D001658), tenderness (MESH:D063806), intrahepatic bile duct dilatation (MESH:C531647), thrombocytosis (MESH:D013922)
- **Chemicals:** bilirubin (MESH:D001663), Hydroxyurea (MESH:D006918)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

1 figure with captions in the complete paper: https://tomesphere.com/paper/PMC12962618/full.md

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Source: https://tomesphere.com/paper/PMC12962618